Between 1990 and 2002, 96 patients 68 women, 28 men. Kikuchifujimoto disease also known as kikuchi disease or histiocytic necrotizing. Kikuchifujimoto disease is a distinctive syndrome of necrotizing lymphadenitis which was formally described in 1972 by two japanese pathologists, kikuchi and fujimoto. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Kikuchis disease was recognized for the first time as a separate syndrome or disease in japan by kikuchis and fujimoto independently in two separate cases among young. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Kikuchifujimoto disease with lymph node, spleen, and. The trip database provides clinical publications about evidence. It predominantly affects young women and can closely mimic infective and immunological disorders. Kikuchifujimoto disease, is usually a benign selflimiting disease which typically affects young females under the age of 30 years and resolves without treatment within six months. In patients with kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. Although ct findings of kikuchi disease kd, or histiocytic necrotizing lymphadenitis, are reported in several case reports, largescale analysis of the disease has not been undertaken.
Bdca2, a novel plasmacytoid dendritic cellspecific type ii ctype lectin. It is a rare, self limiting, benign form of histiocytic necrotizing lymphadenitis, which can be mistaken for tuberculosis, lymphoma or systemic lupus. Misdiagnosing kfd as lymphoma or systemic lupus erythematosus is not uncommon due to the similarity of clinical and histopathological features of these diseases. Pathology of cat scratch disease dr sampurna roy md. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. It is a benign, selflimited disease that predominantly occurs in young women. Kikuchifujimoto disease, also known as kikuchis disease or histiocytic necrotizing lymphadenitis, is a benign, selflimiting cause of lymphadenopathy, variably associated with systemic symptoms. Kikuchifujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, was initially described as a distinct histologic pattern of lymphadenitis generally associated with a benign selflimited clinical course.
Source national institutes of health nih ophanet, a consortium of european partners, currently defines a condition rare when it affects 1 person per 2,000. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder characterized. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. First described independently by kikuchi 27 and fujimoto 19 in 1972, kikuchifujimoto disease is increasingly recognized in the literature. It has a low recurrence rate of 3% to 4%, 1 3 and only several fatal cases have been reported. Pathogenesis, diagnosis, and management of kikuchi. Introduction kikuchis disease kd is a histiocytic necrotizing lymphadenitis that was described for the first time in japan in 1972 almost simultaneously by kikuchi and fujimoto12. Kikuchis disease is a selflimited necrotizing lymphadenitis that is characterized by cervical lymphadenopathy and fever. Novel markers of normal and neoplastic human plasmacytoid dendritic cells. Histopathology of the involved lymph node differentiate kikuchis disease from several more serious conditions particularly lymphomas, which it may mimic1. Abstract kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a.
Kimuras disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. We report a case of kfd in a 47 yearold man who presented with weight loss, fever, enlarged lymph nodes and elevated serum levels. Kikuchi fujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Histiocytic necrotizing lymphadenitis kikuchifujimoto disease. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. This disease is of unknown etiology, but an infectious etiology has been implicated in the. For language access assistance, contact the ncats public information officer. Kikuchis disease of the mesenteric lymph nodes presenting as acute appendicitis corresponding author seung sam paik, m. Electronic medical records were searched for clinical and laboratory manifestations. The bacteria form filaments up to 10 micrometer or longer. Kikuchis disease of the mesenteric lymph nodes presenting. Kikuchi disease is a benign noncancerous condition of the lymph nodes. A 12yearold female suffered from cervical lymphadenopathy, leukocytopenia, fever and.
We characterized the clinical and ct findings in a large group of patients with kd. We want to help promote all of your pathology related books. Lactate dehydrogenase and alanine aminotransferase concentrations can be raised. While the pathogenesis of kikuchi disease is unknown, the clinical presentation, course, and histologic changes suggest an immune response of t cells and histiocytes to an infectious agent. A reappraisal using novel immunohistochemistry combinations.
The disease is characterised by a unilateral posterior cervical lymphadenopathy. Kikuchi fujimoto disease is a selflimited disease that usually resolves within a few months. American journal of clinical pathology, volume 1, issue 2. However, when it occurs in the context of connective tissue disease, particularly systemic lupus erythematosus sle, it is usually associated with a flareup of the patients symptoms, requiring treatment, and.
Examples of singleuser license include conferences, seminars, presentations, and onetime teaching courses. It is easily seen in tissue sections of the skin, lymph nodes, and conjunctiva, when stained by a silver impregnation technique. They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. Kikuchi disease is an idiopathic, generally selflimiting cause for lymphadenitis that can be clinically and histologically mistaken for lymphoma or systemic lupus erythematosus. Department of pathology, college of medicine, hanyang university, 17 haengdangdong, seongdonggu, seoul 3792, korea tel. Clinical studies of kikuchis disease full text view. It was more common in asian people, especially in japanese. The following cases have burn reported for clinical interest and for the rarity of. Complete blood cell cbc count findings include the following. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Successful treatment of severe kikuchis disease with.
Jeffrey medeiros, in diagnostic surgical pathology of the head and neck second edition, 2009. Histiocytic necrotizing lymphadenitis, the socalled kikuchifujimoto disease, was first described in 1972 by two independent japanese pathologists, kikuchi and fujimoto 12 3 is a rare. Fineneedle aspiration and excision biopsy were undertaken. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. Kikuchi disease kd also known as kikuchifujimoto disease, necrotizing histiocytic lymphadenopathy is an uncommon, benign, enigmatous, selflimiting, rarely fatal, disease of young caucasian females, presenting with firm, tender, unilateral cervical lymphadenopathy.
The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients. The clinical features are such that it is often mistaken for infectious. Kikuchifujimoto disease radiology reference article. They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by. This means that kikuchi disease, or a subtype of kikuchi disease, affects less than 200,000 people in the us population. Kikuchifujimoto disease kfd is an extremely rare disease with a worldwide distribution and higher prevalence in asians.
Kikuchi disease symptoms, diagnosis, treatments and causes. Kikuchifujimoto disease kfd, necrotizing lymphadenitis is defined pathologically by enlarged lymph nodes with characteristic microscopic findings and nearly always follows a selflimiting benign clinical course. It is also known as histiocytic necrotising lymphadenitis, abbreviated hnl, and kikuchifujimoto disease. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2.
Ultrasonographic differentiation between kikuchis disease. Cat scratch disease csd is usually a selflimited infection caused by a curved gramnegative, bacteria bartonella henselae. Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. If you have problems viewing pdf files, download the latest version of adobe reader. Kikuchi first described the disease in 1972 in japan. It is a benign and selflimiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Kikuchi fujimoto disease kfd was first described in japan in 1972 almost simultaneously by kikuchi and fujimoto. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. Differentiating this disease from common lymphatic disorder is extremely important from the pathologists point of view, which is highlighted in the article. While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been reported in patients of virtually any age, gender. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo.
Clinical examination revealed enlarged rightsided lymph nodes in the neck. Ferry, in diagnostic pathology of infectious disease second edition, 2018. Kikuchi disease genetic and rare diseases information. Kikuchis disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. Initially described in japan and other asian countries. Kikuchifujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. Find the top 100 most popular items in amazon books best sellers. Kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. A rare cause of fever and lymphadenopathy article pdf available in clinical medicine insights. Fineneedle aspiration cytology of kikuchi fujimoto disease. A handful of fatal cases have been described in addition to sparse reports of empirical treatment with a diverse selection of agents, which include antimicrobial and anti. Kikuchis disease, lymphadenitis, lymphoma, systemic lupus erythematosus.
Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. Kikuchi disease is characterized histopathologically by. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, kikuchi lymphadenitis, kikuchifujimoto disease, and. Histopathology of the involved lymph nodes differentiates kikuchi disease from several more serious conditions that it may mimic. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease. Further radiological scans showed a mass near the carotid and enlarged level v lymph nodes. Characteristic findings on pathology include the presence of crescenteric. Although results of fineneedle aspiration fna of an affected lymph node may be suggestive, 30, 31 the diagnosis of kikuchi disease is confirmed only by excisional lymph node biopsy. Study design this was a retrospective study of children diagnosed with kikuchifujimoto disease from march 2003 to february 2015 in south korea. Kikuchi disease, is a rare pathology of the lymph nodes.
The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. Histologically, the dermatological manifestations of kikuchifujimoto disease have features in common with systemic lupus erythematosus. Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Furthermore, signs and symptoms of kikuchi disease may vary on an individual basis for each patient. This signs and symptoms information for kikuchi disease has been gathered from various sources, may not be fully accurate, and may not be the full list of kikuchi disease signs or kikuchi disease symptoms. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy.
It is rare in caucasians, normally occurring in those of asian descent. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. Kd is a selflimited necrotizing lymphadenitis and occurs predominantly in females between 20 and 30 years old. Results among 86 histopathologically confirmed cases, the mean age was. Kikuchifujimoto disease kfd is a histiocytic necrotising lymphadenitis, which is a benign disease of unknown aetiology.